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Medline ® Abstract for Reference 13

of 'Atypical fibroxanthoma'

Atypical fibroxanthoma and squamous cell carcinoma of the conjunctiva in xeroderma pigmentosum.
Shao L, Newell B, Quintanilla N
Pediatr Dev Pathol. 2007 Mar;10(2):149-52.
Patients with xeroderma pigmentosum (XP) have defective DNA repair and a high predisposition to developing abnormalities and neoplasia in the sun-exposed areas of the skin and mucous membranes. The most common tumors reported in patients with XP are squamous cell carcinomas, basal cell carcinomas, and melanomas. Atypical fibroxanthoma (AFX) is a pleomorphic tumor that arises predominantly in the sun-damaged skin of the head and neck regions of the elderly. We describe a unique case of a 6-year-old African American boy with XP who developed an atypical fibroxanthoma and 2 squamous cell carcinomas in the conjunctiva. The clinical and histopathologic findings of AFX are discussed.
Department of Pathology, Children's Mercy Hospitals and Clinics, 2401 Gillham Road, Kansas City, MO 64108, USA. lshao@cmh.edu